Abstract
Imaging abnormalities are seen in 20% of patients with a congenital sensorineural hearing loss. Malformations can involve the cochlea, vestibule, semicircular canals or internal auditory canal alone or in combination. Incomplete partition II (IP-II) is synonymous with a Mondini’s deformity. The severity of the malformation generally depends on the timing of the gestational development arrest.
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References
Baek SK, Chae SW, Jung HH (2003) Congenital internal auditory canal stenosis. J Laryngol Otol 117(10):784–787
Daneshi A, Farhadi M, Asghari A, Emamjomeh H, Abbasalipour P, Hasanzadeh S (2002) Three familial cases of Michel’s aplasia. Otol Neurotol 23(3):346–348
Guirado CR (1992) Malformations of the inner auditory canal. Rev Laryngol Otol Rhinol (Bord) 113(5):419–421
Kitazawa K, Matsumoto M, Senda M, Honda A, Morimoto N, Kawashiro N, Imashuku S (2004) Mondini dysplasia and recurrent bacterial meningitis in a girl with relapsing Langerhans cell histiocytosis. Pediatr Blood Cancer 43(1):85–87
Koyama S, Iino Y, Kaga K, Ogawa Y (1998) Facial nerve anomalies of children with congenital anomalies [Japanese]. Nippon Jibiinkoka Gakkai Kaiho 101(2):192–197
Paksoy Y, SEker M, Kalkan E (2004) Klippel–Feil syndrome associated with persistent trigeminal artery. Spine 29(9):E193–E196
Satar B, Mukherji SK, Telian SA (2003) Congenital aplasia of the semicircular canals. Otol Neurotol 24(3):437–446
Sennaroglu L, Saatci I (2002) A new classification for cochleovestibular malformations. Laryngoscope 112(12):2230–2241
Sheykholeslami K, Schmerber S, Habiby Kermany M, Kaga K (2004) Vestibular-evoked myogenic potentials in three patients with large vestibular aqueduct. Hearing Res 190(1–2):161–168
Triglia JM, Nicollas R, Ternier F, Cannoni M (1993) Deafness caused by malformation of the inner ear. Current contribution of x-ray computed tomography [French]. Ann Otolaryngol Chir Cervicofac 110(5):241–246
Tullu MS, Khanna SS, Kamat JR, Kirtane MV (2004) Mondini dysplasia and pyogenic meningitis. Indian J Pediatr 71(7):655–657
Zheng Y, Schachern PA, Cureoglu S, Mutlu C, Dijalilian H, Paparella MM (2002) The shortened cochlea: its classification and histopathologic features. Int J Pediat Otorhinolaryngol 63(1):29–39
Malekpour M, Shahidi A, Khorsandi Ashtiani MT, Motasaddi Zarandy M (2007) Novel syndrome of cataracts,retinitis pigmentosa, late onset deafness and sperm abnormalities. Am J Med Genet A 143A:1646–1652
Zarandy MM (2008) Transmastoid labyrinthotomy approach for cochlear implantation in a common cavity malformation. Ear Nose Throat J 87(6):E1–E3
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Zarandy, M.M., Rutka, J. (2010). Anomalies of the Inner Ear. In: Motasaddi Zarandy, M., Rutka, J. (eds) Diseases of the Inner Ear. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-05058-9_3
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DOI: https://doi.org/10.1007/978-3-642-05058-9_3
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